RRC ID 1857
Author Inoue S, Ishikawa K, Nakada K, Sato A, Miyoshi H, Hayashi J.
Title Suppression of disease phenotypes of adult mito-mice carrying pathogenic mtDNA by bone marrow transplantation.
Journal Hum Mol Genet
Abstract For directly addressing the issue of gene therapy of adult patients with mitochondrial diseases, we carried out bone marrow transplantation to adult mito-mice with mutated mtDNA and expressing respiration defects for improvement of disease phenotypes. We supposed that bone marrow cells transdifferentiated into various tissues, so that their transplantation would suppress disease phenotypes. The results showed improvement of survival and delayed expression of renal failure. As most mito-mice without a transplant died due to renal failure, we examined whether transplanted bone marrow cells transdifferentiated into renal tissues carrying improved renal function. Histochemical analyses showed that the suppression of disease phenotypes was not due to transdifferentiation, but due to suppression of apoptosis of renal cells. Thus, bone marrow cells possess a novel function of supporting tissues by suppressing apoptosis induced by respiration defects.
Volume 15(11)
Pages 1801-7
Published 2006-6-1
DOI 10.1093/hmg/ddl102
PII ddl102
PMID 16613898
MeSH Animals Apoptosis Bone Marrow Transplantation / methods* Cell Differentiation DNA, Mitochondrial* / metabolism Genetic Therapy / methods Immunohistochemistry In Situ Nick-End Labeling Male Mice Mice, Transgenic Mutation Phenotype Renal Insufficiency / metabolism
IF 4.544
Times Cited 7
Mice C57BL/6-Tg(CAG-EGFP)C14-Y01-FM131Osb